HELLP syndrome

Other Names: Hemolysis, Elevated Liver Enzymes, Lowered Platelets

HELLP syndrome is a life-threatening condition that can potentially complicate pregnancy. It is named for 3 features of the condition: Hemolysis, Elevated Liver enzyme levels, and Low Platelet levels. It typically occurs in the last 3 months of pregnancy (the third trimester) but can also start soon after delivery.

Cause

The cause of HELLP syndrome is unclear. Although it is more common in women who have preeclampsia or pregnancy induced hypertension (high blood pressure during pregnancy), some women develop HELLP syndrome without showing signs of these conditions.

The following risk factors may increase a woman's chance to develop HELLP syndrome:

• Having a previous pregnancy with HELLP syndrome
• Having preeclampsia or pregnancy induced hypertension
• Being over age 25
• Being Caucasian
• Multiparous (given birth 2 or more times)

In less than 2 percent of women with HELLP syndrome, the underlying cause appears to be related to LCHAD deficiency in the fetus.

Inheritance

A variety of genetic factors (both in the mother and fetus) have been found to play a role in the development of preeclampsia and HELLP syndrome. However, the condition is likely multifactorial. This means that several genetic and environmental factors likely interact to cause HELLP syndrome, and no one gene is thought to be responsible for the condition.

Some women may have a genetic predisposition to developing preeclampsia and related conditions, such as HELLP syndrome. This means that certain genetic factors increase a woman's risk to develop HELLP syndrome. However, many women with a genetic predisposition will never develop HELLP syndrome.

Signs and symptoms

Women with HELLP syndrome may feel tired, have pain in the upper right part of the belly, have bad headaches, and nausea or vomiting. They may also experience swelling, especially of the face and hands. Vision problems may also be observed. Rarely, they may have bleeding from the gums or other places.

Because healthy pregnant women may also have these symptoms late in pregnancy, it may be hard to know for sure if they are attributable to HELLP syndrome. A doctor may order blood tests to determine if these symptoms are the result of HELLP syndrome.

Diagnosis

During a physical exam, the health care provider may discover:

• Abdominal tenderness, especially in the right upper side
• Enlarged liver
• High blood pressure
• Swelling in the legs
• Liver function tests (liver enzymes) may be high. Platelet counts may be low. A CT scan may show bleeding into the liver. Excessive protein may be found in the urine.

Tests of the baby's health will be done. Tests include fetal non-stress test and ultrasound, among others.

Treatment

The main treatment is to deliver the baby as soon as possible, even if the baby is premature. Problems with the liver and other complications of HELLP syndrome can quickly get worse and be harmful to both the mother and child. Your provider may induce labor by giving you medicines to start labor, or may perform a C-section.

You may also receive:

• A blood transfusion if bleeding problems become severe
• Corticosteroid medicines to help the baby's lungs develop faster
• Medicines to treat high blood pressure
• Magnesium sulfate infusion to prevent seizures

Prognosis

The long-term outlook (prognosis) for mothers with HELLP syndrome varies from woman to woman, but HELLP syndrome is rarely fatal. The outcome for mothers with HELLP is generally good, but serious complications are relatively common. In most cases, after delivery, a mother's signs and symptoms begin to improve within 2 days. However, some women experience complications. The risk of serious complications generally is higher for women with severe symptoms and for those with more severe laboratory abnormalities. Examples of complications include disseminated intravascular coagulation (DIC), placental abruption, acute kidney failure, and pulmonary edema. However, these complications are related. For example, placental abruption can cause DIC, which then may cause acute kidney failure; acute kidney failure may lead to pulmonary edema. Of note, HELLP syndrome with or without kidney failure does not affect long-term kidney function. Other complications that have been reported include adult respiratory distress syndrome, sepsis, and stroke.

The prognosis for a fetus or newborn of a mother with HELLP syndrome is thought to largely depend on the gestational age at delivery and birth weight (rather than the fact that HELLP syndrome was present, or the severity of signs and symptoms). This means that in many cases, the longer the pregnancy continues, the better the chance for survival and good health for the baby. Premature birth is common in mothers with HELLP syndrome. The rate of preterm delivery is 70%, with 15% occurring before 28 weeks of gestation. This is the leading cause of fetal or newborn death, with the overall risk of death ranging from 7 to 20 percent.

The mother's laboratory abnormalities are not thought to be related to the chance of survival of a fetus or newborn. Additionally, HELLP syndrome does not affect liver function in the fetus or newborn. Unfortunately, there is very limited data available regarding the long-term follow-up of children born to mothers with HELLP syndrome.

Possible Complications There can be complications before and after the baby is delivered, including:

• Disseminated intravascular coagulation (DIC). A clotting disorder that leads to excess bleeding (hemorrhage).
• Fluid in the lungs (pulmonary edema)
• Kidney failure
• Liver hemorrhage and failure
• Separation of the placenta from the uterine wall (placental abruption)
• After the baby is born, HELLP syndrome goes away in most cases.

NIH genetic and rare disease info

• NIH info on HELLP syndrome

HELLP syndrome is a rare disease.

HELLP syndrome Resources
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